AB0927 SYSTEMIC SCLERODERMA: ABOUT 60 CASES
Authors: A. Taouch, W. Ammouri, F. Ibourk el Idrissi, W. Chawad, N. Mouatassim, H. Khibri, M. Maamar, H. Harmouche, Z. Tazi Mezalek, M. Adnaoui
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Systemic scleroderma is a relatively rare and potentially serious and debilitating connectivitis. It also seems to be different from other diseases in this group, by its pathogenic process that involves several successive or concurrent disorders involving fibroblastic, vascular and dysimmune phenomena. Some impairments condition the functional prognosis and may affect the life-threatening prognosis. <h3>Methods</h3> We conducted a 10-year, single-centric retrospective study (2011 and 2021) of 60 patients followed up for systemic scleroderma at the Internal Medicine Department of the Ibn Sina Hospital in Rabat. We analyzed the statistical data using JAMOVI 2. 2. 5. <h3>Results</h3> We collected 60 files. The sex ratio (F/H) was 5 (50 women/ 10 men), with a mean age of 51±12. 6 (26-76 years). The mean time to diagnosis was 9. 5±38. 9 months (1–180 months). General signs were present in 23 cases (38. 3%) Skin involvement was constant. Raynaud's syndrome was reported in 49 cases (81. 7%) sclerodactyly in 23 cases (38. 3), trophic disorders in 19 cases (31. 7%), telangiectasis in 13 cases (21. 7%) and subcutaneous calcinosis in 5 cases (8. 3%). Rodnan's modified score at admission ranged from 1 to 47/51. Joint manifestations were noted in 36 patients (60%). Lung involvement was objectified in 40 cases (66. 7%), revealing in 22 cases (36%). Interstitial syndrome was observed in 13 cases (21. 7%). Respiratory functional explorations revealed a restrictive syndrome in 23 cases (40%). Chest CT showed diffuse interstitial lung disease in 29 cases (48. 3%), pulmonary fibrosis in 12 cases (20. 3%). Cardiac involvement was detected in 26 cases (43. 3%): pulmonary arterial hypertension in 20 cases (33. 3%); pericarditis in 5 cases (8. 3%); heart failure in 5 cases (8. 3%); and rhythm disturbance in 1 case. Digestive involvement was noted in 17 patients (28. 3%), dominated by oesophageal involvement in all cases, associated with gastric involvement in 6 cases (10%). duodenal garstro esophageal fibroscopy objectified esophagitis and its complications in 17 cases (28. 3%). Neurological involvement was present in 3 cases. Kidney damage was absent from our series. Inflammatory syndrome was present in 37 cases. Anti-nuclear antibodies were positive in 42 cases, anti-Scl70 in 28 cases, anti-centromere in 5 cases, anti-RNP in 6 cases and anti-PM-Scl in 6 cases. An association with another autoimmune disease was noted in 18 patients: Gougerot Sjorgen Syndrome was noted in 5 cases, rheumatoid arthritis in 5 cases, disseminated lupus erythematosus in 5 cases, anti-phospholipid syndrome in 4 cases, primary biliary cirrhosis in 2 cases, hashimoto's thyroiditis in 2 cases, inflammatory myopathy in 1 case and Bullous linear dermatosis to igA in 1 case. Neoplasmic pathology occurred during progression in 3 cases: 1 case of marginal lymphoma and 2 cases of lung cancer. Colchicine was prescribed in 24 cases, D-Penicillamine in 1 case, calcium inhibitors in 31 cases. Corticosteroid therapy was prescribed in 29 cases. Forty-five patients received immunosuppressive therapy and three patients received anti-CD20 (Rituximab) for one patient indicated for rheumatoid arthritis. Complications of immunosuppressive therapy were predominantly infection and impaired liver function. The outcome was favourable in the majority of patients. Late relapses &gt; 6 months were reported in 12 patients, including 8 pulmonary relapses, 1 skin relapses and 1 muscle relapses. The outcome of lung disease was unfavourable in 3 cases. And six patients died. We analyzed several associations such as: lung and heart disease, lung and digestive disease, and heart and digestive disease, the only significant revenue was the association of lung and cardiac disease that was significant (p = 0. 025) <h3>Conclusion</h3> The results of our study are consistent with the literature and confirm the predominance of women and the relatively high incidence of skin, joint, lung and cardiac involvement. Vital prognosis is conditioned by severe visceral damage, especially pulmonary and cardiac. <h3>REFERENCES:</h3> NIL. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.